Cargando…

Strategies to improve the quality of life in patients with hereditary transthyretin amyloidosis (hATTR) and autonomic neuropathy

PURPOSE: Hereditary transthyretin amyloidosis (hATTR) is a severe adult-onset progressive disease mainly involving the peripheral nervous system and the heart, with a prominent impact on the autonomic nervous system. This review summarizes the clinical aspects of autonomic dysfunction in hATTR, and...

Descripción completa

Detalles Bibliográficos
Autores principales: Gendre, Thierry, Planté-Bordeneuve, Violaine
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6763624/
https://www.ncbi.nlm.nih.gov/pubmed/31506870
http://dx.doi.org/10.1007/s10286-019-00624-w
_version_ 1783454237152247808
author Gendre, Thierry
Planté-Bordeneuve, Violaine
author_facet Gendre, Thierry
Planté-Bordeneuve, Violaine
author_sort Gendre, Thierry
collection PubMed
description PURPOSE: Hereditary transthyretin amyloidosis (hATTR) is a severe adult-onset progressive disease mainly involving the peripheral nervous system and the heart, with a prominent impact on the autonomic nervous system. This review summarizes the clinical aspects of autonomic dysfunction in hATTR, and their impact on quality of life as well as potential therapeutic options. METHODS: Literature review. RESULTS: Autonomic dysfunction, causing neurogenic orthostatic hypotension, gastroparesis, constipation, diarrhea, bladder dysfunction, and erectile dysfunction in males, has a major impact on the quality of life of patients with hATTR. Improvement of qualify of life in patients with hATTR implies periodic symptomatic screening and early management, taking into consideration comorbidities and medication side effects. The specific effect of the disease-modifying treatment on this aspect remains to be unraveled. CONCLUSIONS: Management of autonomic dysfunction in patients with hAATR is feasible and can result in improved qualify of life. Novel disease-modifying treatments for hAATR may contribute to improve autonomic dysfunction, although specific studies are required.
format Online
Article
Text
id pubmed-6763624
institution National Center for Biotechnology Information
language English
publishDate 2019
publisher Springer Berlin Heidelberg
record_format MEDLINE/PubMed
spelling pubmed-67636242019-10-07 Strategies to improve the quality of life in patients with hereditary transthyretin amyloidosis (hATTR) and autonomic neuropathy Gendre, Thierry Planté-Bordeneuve, Violaine Clin Auton Res Review PURPOSE: Hereditary transthyretin amyloidosis (hATTR) is a severe adult-onset progressive disease mainly involving the peripheral nervous system and the heart, with a prominent impact on the autonomic nervous system. This review summarizes the clinical aspects of autonomic dysfunction in hATTR, and their impact on quality of life as well as potential therapeutic options. METHODS: Literature review. RESULTS: Autonomic dysfunction, causing neurogenic orthostatic hypotension, gastroparesis, constipation, diarrhea, bladder dysfunction, and erectile dysfunction in males, has a major impact on the quality of life of patients with hATTR. Improvement of qualify of life in patients with hATTR implies periodic symptomatic screening and early management, taking into consideration comorbidities and medication side effects. The specific effect of the disease-modifying treatment on this aspect remains to be unraveled. CONCLUSIONS: Management of autonomic dysfunction in patients with hAATR is feasible and can result in improved qualify of life. Novel disease-modifying treatments for hAATR may contribute to improve autonomic dysfunction, although specific studies are required. Springer Berlin Heidelberg 2019-09-10 2019 /pmc/articles/PMC6763624/ /pubmed/31506870 http://dx.doi.org/10.1007/s10286-019-00624-w Text en © The Author(s) 2019 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.
spellingShingle Review
Gendre, Thierry
Planté-Bordeneuve, Violaine
Strategies to improve the quality of life in patients with hereditary transthyretin amyloidosis (hATTR) and autonomic neuropathy
title Strategies to improve the quality of life in patients with hereditary transthyretin amyloidosis (hATTR) and autonomic neuropathy
title_full Strategies to improve the quality of life in patients with hereditary transthyretin amyloidosis (hATTR) and autonomic neuropathy
title_fullStr Strategies to improve the quality of life in patients with hereditary transthyretin amyloidosis (hATTR) and autonomic neuropathy
title_full_unstemmed Strategies to improve the quality of life in patients with hereditary transthyretin amyloidosis (hATTR) and autonomic neuropathy
title_short Strategies to improve the quality of life in patients with hereditary transthyretin amyloidosis (hATTR) and autonomic neuropathy
title_sort strategies to improve the quality of life in patients with hereditary transthyretin amyloidosis (hattr) and autonomic neuropathy
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6763624/
https://www.ncbi.nlm.nih.gov/pubmed/31506870
http://dx.doi.org/10.1007/s10286-019-00624-w
work_keys_str_mv AT gendrethierry strategiestoimprovethequalityoflifeinpatientswithhereditarytransthyretinamyloidosishattrandautonomicneuropathy
AT plantebordeneuveviolaine strategiestoimprovethequalityoflifeinpatientswithhereditarytransthyretinamyloidosishattrandautonomicneuropathy